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DOI: 10.1055/s-0039-1692409
Prognostic Factors for Long-Term Survival after Surgical Resection of Primary Cardiac Sarcoma
Publikationsverlauf
20. Februar 2019
29. April 2019
Publikationsdatum:
27. Juni 2019 (online)
![](https://www.thieme-connect.de/media/thoracic/201908/lookinside/thumbnails/10-1055-s-0039-1692409_195504-1.jpg)
Abstract
Background Primary cardiac sarcoma (CS) is an extremely rare disease. This study aims to identify possible prognostic factors for long-term survival.
Methods A total of 17 consecutive patients who were treated for primary CS between 2003 und 2018 at two cardiac centers were investigated. Clinical data and histological characteristics of the tumors were analyzed. Long-term follow-up of all patients were performed.
Results The median age was 54 years (range: 23–74). The tumors originated from the left side of the heart in nine patients. Histologically, there were four angiosarcomas, three intimal sarcomas, and three synovial sarcomas. One- and 7-year survivals were 81.9 and 18.2%, respectively. Low expression levels of Ki-67 tended to be associated with increased survival (log-rank p = 0.06). Adjuvant chemotherapy but not radiotherapy regardless of existing metastases was associated with significantly increased survival (log-rank p = 0.001).
Conclusion Angiosarcoma was the most common type of CS. The survival of CS patients is poor but prognostic factors, such as Ki-67, may help estimate the course of the disease. Survival could be improved significantly with chemotherapy.
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References
- 1 Straus R, Merliss R. Primary tumors of the heart. Arch Pathol (Chic) 1945; 39: 74-78
- 2 Kim CH, Dancer JY, Coffey D. , et al. Clinicopathologic study of 24 patients with primary cardiac sarcomas: a 10-year single institution experience. Hum Pathol 2008; 39 (06) 933-938
- 3 Castillo JG, Silvay G. Characterization and management of cardiac tumors. Semin Cardiothorac Vasc Anesth 2010; 14 (01) 6-20
- 4 Look Hong NJ, Pandalai PK, Hornick JL. , et al. Cardiac angiosarcoma management and outcomes: 20-year single-institution experience. Ann Surg Oncol 2012; 19 (08) 2707-2715
- 5 Burazor I, Aviel-Ronen S, Imazio M. , et al. Primary malignancies of the heart and pericardium. Clin Cardiol 2014; 37 (09) 582-588
- 6 Patel SD, Peterson A, Bartczak A. , et al. Primary cardiac angiosarcoma - a review. Med Sci Monit 2014; 20: 103-109
- 7 Ramlawi B, Leja MJ, Abu Saleh WK. , et al. Surgical treatment of primary cardiac sarcomas: review of a single-institution experience. Ann Thorac Surg 2016; 101 (02) 698-702
- 8 Glancy DL, Morales Jr JB, Roberts WC. Angiosarcoma of the heart. Am J Cardiol 1968; 21 (03) 413-419
- 9 Janigan DT, Husain A, Robinson NA. Cardiac angiosarcomas. A review and a case report. Cancer 1986; 57 (04) 852-859
- 10 Butany J, Yu W. Cardiac angiosarcoma: two cases and a review of the literature. Can J Cardiol 2000; 16 (02) 197-205
- 11 Hoffmeier A, Sindermann JR, Scheld HH, Martens S. Cardiac tumors--diagnosis and surgical treatment. Dtsch Arztebl Int 2014; 111 (12) 205-211
- 12 Leduc C, Jenkins SM, Sukov WR, Rustin JG, Maleszewski JJ. Cardiac angiosarcoma: histopathologic, immunohistochemical, and cytogenetic analysis of 10 cases. Hum Pathol 2017; 60: 199-207
- 13 Lam KY, Dickens P, Chan AC. Tumors of the heart. A 20-year experience with a review of 12,485 consecutive autopsies. Arch Pathol Lab Med 1993; 117 (10) 1027-1031
- 14 Mayer F, Aebert H, Rudert M. , et al. Primary malignant sarcomas of the heart and great vessels in adult patients--a single-center experience. Oncologist 2007; 12 (09) 1134-1142
- 15 Burke A, Jeudy Jr J, Virmani R. Cardiac tumours: an update: cardiac tumours. Heart 2008; 94 (01) 117-123
- 16 Kumar N, Agarwal S, Ahuja A, Das P, Airon B, Ray R. Spectrum of cardiac tumors excluding myxoma: experience of a tertiary center with review of the literature. Pathol Res Pract 2011; 207 (12) 769-774
- 17 Miettinen M. Immunohistochemistry of soft tissue tumours - review with emphasis on 10 markers. Histopathology 2014; 64 (01) 101-118
- 18 Sorbye SW, Kilvaer TK, Valkov A. , et al. Prognostic impact of CD57, CD68, M-CSF, CSF-1R, Ki67 and TGF-beta in soft tissue sarcomas. BMC Clin Pathol 2012; 12: 7
- 19 Sorbye SW, Kilvaer TK, Valkov A. , et al. Prognostic impact of Jab1, p16, p21, p62, Ki67 and Skp2 in soft tissue sarcomas. PLoS One 2012; 7 (10) e47068
- 20 Levenson RM, Borowsky AD, Angelo M. Immunohistochemistry and mass spectrometry for highly multiplexed cellular molecular imaging. Lab Invest 2015; 95 (04) 397-405
- 21 Pucci A, Gagliardotto P, Zanini C, Pansini S, di Summa M, Mollo F. Histopathologic and clinical characterization of cardiac myxoma: review of 53 cases from a single institution. Am Heart J 2000; 140 (01) 134-138
- 22 Li LT, Jiang G, Chen Q, Zheng JN. Ki67 is a promising molecular target in the diagnosis of cancer (review). Mol Med Rep 2015; 11 (03) 1566-1572
- 23 Fletcher C, Unni K, Mertens F. Pathology and genetics of tumors of soft tissue and bone. World Health Organization Classification of Tumors. Lyon: IARC Press; 2002: 10-18
- 24 Kaplan E, Meier P. Nonparametric estimation from incomplete observations. J Am Stat Assoc 1958; 53: 457-481
- 25 Putnam Jr JB, Sweeney MS, Colon R, Lanza LA, Frazier OH, Cooley DA. Primary cardiac sarcomas. Ann Thorac Surg 1991; 51 (06) 906-910
- 26 Li H, Yang S, Chen H. , et al. Survival after heart transplantation for non-metastatic primary cardiac sarcoma. J Cardiothorac Surg 2016; 11 (01) 145
- 27 Eckstein R, Gössner W, Rienmüller R. Primary malignant fibrous histiocytoma of the left atrium. Surgical and chemotherapeutic management. Br Heart J 1984; 52 (03) 354-357